Simmonds Nicholas J, Wyatt Hilary, Patel Raj, Hodson Margaret E, Gyi Khin M
J Med Case Rep. 2009 Jul 20;3:8582. doi: 10.4076/1752-1947-3-8582.
Cystic fibrosis is usually associated with chronic pulmonary sepsis and frequent infective exacerbations. We report a very unusual cause of severe hypoxaemia in a woman with cystic fibrosis caused by thrombus formation in the right atrium.
A 21-year-old Caucasian woman with cystic fibrosis and a totally implantable venous access device presented with severe hypoxaemia. This was initially treated with antibiotics but her oxygen levels did not improve significantly. Subsequently, a transient ischaemic attack occurred. Further investigations, including a contrast echocardiogram and a cardiac magnetic resonance scan, revealed the presence of a large right atrial thrombus and right-to-left intracardiac shunt through a patent foramen ovale.
This case highlights the need to consider a right-to-left shunt in chronic respiratory diseases when hypoxaemia is out of proportion to the degree of lung function impairment. Totally implantable venous access devices should always be considered as a source of thrombus formation.
囊性纤维化通常与慢性肺部感染和频繁的感染性加重相关。我们报告了一名患有囊性纤维化的女性因右心房血栓形成导致严重低氧血症的非常罕见的病因。
一名21岁患有囊性纤维化且植入了完全植入式静脉通路装置的白种女性出现严重低氧血症。最初用抗生素治疗,但她的氧水平没有明显改善。随后,发生了一次短暂性脑缺血发作。进一步检查,包括对比超声心动图和心脏磁共振扫描,发现存在一个大的右心房血栓以及通过卵圆孔未闭的右向左心内分流。
该病例强调,当低氧血症与肺功能损害程度不成比例时,在慢性呼吸系统疾病中需要考虑右向左分流。完全植入式静脉通路装置应始终被视为血栓形成的一个来源。